Approach Considerations

The management of Tourette syndrome (TS) is multifaceted. The approach is primarily aimed at medical management of frequent or disabling tics, treatment of coexisting behavior symptoms, and patient and family education.

Ideally, patients with mild tics who have made a good adaptation in their lives can avoid the use of medications. Educating patients, family members, peers, and school personnel regarding the nature of TS; restructuring the school environment; and providing supportive counseling are measures that may be sufficient to avoid pharmacotherapy. Information is available through school counselors, psychologists, representatives of local chapters of the Tourette Syndrome Association, or related topical organizations.

Pharmacologic therapy for tics is considered when tics interfere with social interactions, school performance, or activities of daily living. The goal of such therapy is not complete elimination of the tic, but rather control of tics to alleviate the social embarrassment or discomfort due to the tic, therefore improving social functioning.

Various therapeutic agents are now available to treat patients with tics, and each medication should be chosen on the basis of expected efficacy and potential adverse effects. Dosages should be titrated slowly to achieve the lowest satisfactory dosage that is sufficient to attain a tolerable level of symptoms. (See Medication)

Only rarely do patients with TS need hospitalization. Most of the patients who do require hospitalization have comorbid conditions and are a threat to themselves or to others. Patients with the complex tics of coprolalia or copropraxia might need a brief hospitalization if their families have difficulty controlling them.

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Treatment of Tics

The alpha2-adrenergic drugs clonidine and guanfacine are first-line agents in treating mild to moderate tics. Clonidine decreases plasma norepinephrine levels and can also reduce the symptoms of attention deficit–hyperactivity disorder (ADHD). The daily dosage range for clonidine is 0.1-0.3 mg in divided doses.

Guanfacine, with a similar mechanism of action as clonidine, has a longer half-life and may treat ADHD symptoms not responsive to clonidine. For guanfacine, the daily dosage range is 0.5-3.0 mg in divided doses. Clonazepam and baclofen can be considered first-line alternatives as well.

The D2 dopamine receptor—blocking medications (neuroleptics) are the most effective medications for treating tics, and many experts use the neuroleptics as the initial agent of choice for that reason. However, the side effect profile, which includes extrapyramidal symptoms/tardive dyskinesia, is often a limitation to using these agents as first-line therapy.

Haloperidol and pimozide, the 2 most studied neuroleptics, have been approved for the treatment of tics in TS by the US Food and Drug Administration (FDA). Well-controlled clinical trials indicate that haloperidol has a response rate approaching 80% for tic suppression.

More recently, the atypical neuroleptics that interact with both serotonin and dopamine receptors and have less extrapyramidal effects have been shown to be effective in suppressing tics. Out of this group, risperidone has been the most studied, showing efficacy equal to that of clonidine. Olanzapine, ziprasidone, and quetiapine have shown promise in small studies.

Aripiprazole is approved by the FDA for pediatric Tourette syndrome. Aripiprazole elicits partial agonistic effect at dopamine D2 and serotonin type 1 (5-HT1A) receptors (thought to help control vocal tics). It also shows antagonist effect at serotonin type 2 (5-HT2A) receptor, which may be helpful in managing some of the comorbidities of Tourette syndrome (e.g., obsessional traits, depression). Its approval was based on an 8-week and 10-week study. In each study, patients taking aripiprazole demonstrated improvement compared to patients taking placebo. The improvement was measured by the Total Tic Score, the Tourette's Syndrome Clinical Global Impression-Improvement Scale, and the Yale Global Tic Severity Scale.
[13, 14]

Tetrabenazine, a drug that blocks dopamine and depletes catecholamines at presynaptic terminals may be a potent drug in suppressing tics. Low doses of the dopamine agonist ropinirole have been shown to improve tics in small studies. The dopamine agonist pergolide, although effective, was withdrawn from the US market on March 29, 2007. Botulinum toxin-A has been effective in treating motor and vocal tics in select patients.

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Treatment of ADHD

The most effective medications for the treatment of ADHD are the central nervous system stimulants. Methylphenidate and dextroamphetamine are first-line agents for the treatment of ADHD. Unfortunately, these medications may increase the frequency and intensity of tics. However, with prolonged use, the initial worsening of tics may stabilize. The dopamine-blocking agents can be used concurrently with the CNS stimulants for tic suppression.

Alpha2-adrenergic blockers may be helpful in controlling tics and treating symptoms of ADHD. Both clonidine and guanfacine can control the symptoms of ADHD and impulse control.

Other medications that can be effective in treating ADHD symptoms without worsening tics include bupropion and the tricyclic antidepressants.

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Treatment of OCD in Tourette Syndrome

The selective serotonin reuptake inhibitors (SSRIs) are the most effective treatment of OCD symptoms in TS. This class of drugs includes fluoxetine, fluvoxamine, paroxetine, sertraline, escitalopram, and citalopram. Clomipramine is also effective due to its serotonin reuptake action. Augmentation of SSRIs with atypical antipsychotics may be beneficial in patients with TS and OCD that are poorly responsive to treatment with SSRIs.
[15]

Various psychotherapeutic techniques, including assertiveness training, cognitive therapy, and self-monitoring, have been tried in the treatment of patients with TS.

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Deep Brain Stimulation

Surgical approaches for TS have been attempted in patients who are severely disabled and have inadequate responses to other therapies. Deep brain stimulation (DBS) has been suggested as a potential therapy for severe and disabling tics.
[16] At this time, only isolated cases report the effectiveness of DBS. Patient selection and criteria formation for controlled trials is currently underway.

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Psychotherapeutic Counseling and Support

In addition to patient education, other measures should be taken to nurture self-esteem and self-correction. Individual counseling, cognitive and behavioral therapies, and group therapy should be considered.

Areas of strength should be emphasized, such as talents and skills, interests, any family or peer supports, and psychological resilience. Social skills training can help develop and reinforce more effective methods of confident and prosocial communication. Parents or other guardians may benefit from parent behavior management and discipline training, recognizing that the underlying purpose of discipline is to instill a sense of self-control and responsibility for one's behavior.

Allowances must be made for the child's uncontrollable behaviors that result from the disorder, but some behaviors, such as spitting at others or obscene gestures, have negative social connotations and require special guidance. Methods to help the child manage these behaviors include nonjudgmental acceptance of the child regardless of the nature of the behaviors and working with the child to adapt or substitute alternative, more appropriate behaviors that satisfy premonitory urges, such as spitting into a handkerchief instead of spitting openly.

Parenting skills books, workshops, and trained specialists are widely available and emphasize practical methods in positive reinforcement of desirable behaviors through giving praise or rewards, modeling appropriate behaviors, and administering "time-outs" from rewards or attention for inappropriate or uncontrolled behaviors. Parents also may benefit from group support and education or other topical groups and from individual supportive counseling to cope with accompanying stress.

A local TS support group may be of great benefit to patients and family members. Individual, group, or family counseling may help in facilitating a healthy adaptation to the illness.

Several relaxation or stress management treatment approaches reportedly improve the tics in TS. For example, tics are known to worsen from stress and to improve during periods of relaxation. Whether such therapies have a direct effect on the tics or exert an indirect influence by allowing patients to deal more productively with life stresses is unclear.

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Consultations

Treatment of patients with TS should be a collaborative effort. The neurologist, psychiatrist, psychologist, family members, and school professionals all have important roles.